About Me

My mother was murdered by what I call corporate and political homicide i.e. FOR PROFIT! she died from a rare phenotype of CJD i.e. the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. sporadic, simply meaning from unknown route and source. I have simply been trying to validate her death DOD 12/14/97 with the truth. There is a route, and there is a source. There are many here in the USA. WE must make CJD and all human TSE, of all age groups 'reportable' Nationally and Internationally, with a written CJD questionnaire asking real questions pertaining to route and source of this agent. Friendly fire has the potential to play a huge role in the continued transmission of this agent via the medical, dental, and surgical arena. We must not flounder any longer. ...TSS

OBJECTIVE: To demonstrate that MRI detection of subtle focal cortical
abnormalities can prove more useful than positron emission tomography (PET) in
the diagnosis of Heidenhain variant Creutzfeldt-Jakob Disease (hvCJD).

BACKGROUND: hvCJD is a rare neurodegenerative, spongiform encephalopathy
with an aggressive clinical course. PET brain imaging has been reported to
detect focal cortical abnormalities in hvCJD with greater sensitivity than MRI.
However, because PET is both more costly and less accessable than MRI, early
diagnosis of this disease and subsequent prognostication may be unnecessarily
delayed. The reliability of MRI over PET in detecting isolated occipital
cortical changes suggestive of hvCJD has not been well studied.

DESIGN/METHODS: This is a case report with relevent neuroimaging review.

CONCLUSIONS: hvCJD should be considered in patients with rapid-onset
idiopathic visual disturbance and dementia. When combined with EEG and CSF
analysis, isolated MRI visual cortex diffusion restriction is suggestive of this
ultra-aggressive prion variant. MRI is able to efficiently facilitate valuable
prognostication early in hvCJD and can be more useful than costly PET imaging.

Disclosure: Dr. Beary has nothing to disclose. Dr. Manno has nothing to
disclose.

This subtype is observed in patients who are MM homozygous or MV
heterozygous at codon 129 of the PrP gene (PRNP) and carry PrPSc Type 1.
Clinical duration is short, 3‑4 months.32 The most common presentation in
sCJDMM1 patients is cognitive impairment leading to frank dementia, gait or limb
ataxia, myoclonic jerks and visual signs leading to cortical blindness
(Heidenhain’s syndrome)...

Animals injected with iatrogenic Creutzfeldt–Jakob disease MM1 and genetic
Creutzfeldt–Jakob disease MM1 linked to the E200K mutation showed the same
phenotypic features as those infected with sporadic Creutzfeldt–Jakob disease
MM1 prions...

*** our results raise the possibility that CJD cases classified as VV1 may
include cases caused by iatrogenic transmission of sCJD-MM1 prions or food-borne
infection by type 1 prions from animals, e.g., chronic wasting disease prions in
cervid. In fact, two CJD-VV1 patients who hunted deer or consumed venison have
been reported (40, 41). The results of the present study emphasize the need for
traceback studies and careful re-examination of the biochemical properties of
sCJD-VV1 prions. ***

The epidemiological findings in sCJD demonstrate that approximately 80% of
patients are diagnosed with “classic CJD” types MM1 and MV1, which might
intriguingly suggest an infectious rather than genetic origin for the majority
of sCJD cases.

snip...

Therefore if sCJD(MV2) and sCJD(VV2) were to become iatrogenic sources of
human infection, the host response may be indistinguishable from sCJD(MM1) and
more transmissible with respect to further infection.

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*** A parliamentary inquiry has been launched today into the safety of
blood, tissue and organ screening following fears that vCJD – the human form of
‘mad cow’ disease – may be being spread by medical procedures